Cerebellar Degeneration: An Overview

Cerebellar degeneration is a type of damage to the cerebellum, which is the area of the brain that controls balance and coordination. Cerebellar degeneration is not common. It can occur due to progressive neurological diseases or brain damage, such as a stroke.

Degeneration of the cerebellum causes a type of movement problem called ataxia, which is characterized by poor coordination (waviness) of movement, loss of balance, and difficulty with purposeful movements.

Some types of cerebellar ataxia can be treated, and any type of cerebellar degeneration requires medical care, physical therapy, and supportive devices to help improve motor control and to prevent injuries. 

Symptoms of Cerebellar Degeneration

Degeneration of the cerebellum can cause a variety of symptoms associated with impaired coordination. You can have some or all of the symptoms, and they can develop rapidly or gradually over time. 

Symptoms of cerebellar degeneration include: 

• Nystagmus: Eye movements that appear to be jumping 
• Ataxia: Poorly coordinated movement of the arms, legs, or body when trying to move
• Titubation: The head may appear to bounce or shake while at rest or with movements 
• Ataxic speech: An irregular rhythm when talking
• Diplopia: Double vision
• Vertigo: A feeling that the room is spinning or body is turning, may be accompanied by nausea or vomiting 
• Dizziness: Can occur due to the eye movements 
• Gait imbalance: Difficulty standing, walking, or sitting upright

These symptoms are very noticeable with cerebellar degeneration. This makes it difficult to manage day-to-day life, including things like self-care, eating, walking, climbing stairs, or carrying objects. If cerebellar degeneration progresses, independence declines and can eventually be lost.

Causes

The causes of cerebellar degeneration are varied. While the condition is rare, it can be caused by a hereditary disease, neurodegenerative disease, infections, brain damage, and toxins. What these causes have in common is that they damage cells in the cerebellum.

Sometimes cerebellar degeneration occurs along with other types of brain damage or with systemic (whole-body) disease too. 

Causes include: 

• Stroke: A stroke is brain damage due to a sudden interruption of blood supply in the brain. Cerebellar stroke is not common, but it can occur due to an interruption of blood supply in the cerebellar arteries.
• Alcohol: Alcohol can be toxic to the cerebellar cells, leading to gradual cerebellar degeneration.
• Encephalitis: An infection or inflammation of the brain can affect the cerebellum. Encephalitis is not common, but infectious forms of encephalitis can occur among people who have an impaired immune system.
• Multisystem atrophy (MSA): This degenerative neurological disease causes progressively worsening deterioration of the cerebellum and other regions of the brain. The cause of the condition is unknown. It leads to dementia and ataxia.
• Paraneoplastic syndromes: Certain cancers, particularly ovarian cancer, can cause the production of antibodies (immune proteins) that attack the cerebellum or other parts of the nervous system. Sometimes the neurological symptoms can begin before the cancer is diagnosed.
• Multiple sclerosis (MS): This inflammatory demyelinating (destruction of the protective sheath of nerve fibers) neurological condition affects the brain, spinal cord, and optic nerves, typically with relapses and remissions. The cerebellum is not commonly involved in MS, but it can be affected.
• Creutzfeldt-Jakob disease (CJD): This condition is caused by a prion, which is a type of protein particle that causes damage to the brain. Sometimes the condition is transmitted by contact with contaminated nerve tissue.
• Spinocerebellar ataxia: This rare group of genetic conditions can be hereditary. There are several types, and each causes progressive degeneration of the spinal cord and cerebellum.
• Anatomical abnormalities: Some congenital (from birth) malformations, like Dandy-Walker malformation and chiari malformation, can cause impaired development of the cerebellum. And the defect can cause further cerebellar degeneration over time.

There are also a number of rare cerebellar syndromes—some occur during childhood, and some occur during adulthood. They are often idiopathic (without a known cause), or some may be genetic. An example is cerebellar mutism, which is caused by damage to the pathways between the cerebellum and the cerebral cortex.

Diagnosis

Cerebellar degeneration is diagnosed based on a combination of clinical examination and brain imaging. Additionally, blood tests can help in identifying the cause.

Your evaluation would begin with talking to your healthcare provider about your symptoms and medical history. 

You would have a complete physical examination, including a neurological exam. Some notable physical abnormalities with cerebellar ataxia include nystagmus, poorly coordinated movement, and trouble walking.

Other features, such as muscle strength, sensation, and reflexes, would reflect the underlying cause, such as a stroke or MS. 

You would likely have a brain imaging test, such as magnetic resonance imaging (MRI), which provides good visualization of the cerebellum. You might also have blood tests, such as tests for paraneoplastic antibodies. 

Treatment

Since there are so many diverse causes of cerebellar degeneration, the treatments are varied. Anyone who has cerebellar ataxia, regardless of the cause, could benefit from physical therapy and safety measures to prevent injuries. 

The treatment for cerebellar symptoms of an acute MS exacerbation is typically high-dose steroids. MS disease-modifying therapies (DMTs) are prescribed to prevent further exacerbations and additional declines in neurological function.

Paraneoplastic cerebellar degeneration is treated with surgical removal of the tumor. In many cases, this improves the cerebellar symptoms or prevents the cerebellar effects from worsening. 

Encephalitis may be treated with anti-inflammatory medications. If encephalitis is caused by an infection, treatment that targets the infectious organism, such as antibiotics for a bacterial infection, may help resolve the infection. 

A stroke is a medical emergency, and sometimes immediate treatment with a blood thinner or medical stabilization can minimize the long-term damage. 

When the underlying cause is progressive and cannot be treated, such as MSA and CJD, treatment may include medication to prevent involuntary movements or medications to reduce pain from muscle spasticity. 

Complications

The ataxia caused by cerebellar degeneration can increase your risk of falling and injuries. You can work with your family and healthcare provider to improve your safety and reduce these risks. 

Outlook

The underlying cause of your cerebellar degeneration is the main factor that determines the outlook.

Alcohol-associated cerebellar degeneration can stabilize if alcohol is discontinued, but the effects are not expected to reverse.

Cerebellar degeneration after a stroke should remain stable unless another stroke occurs. Identifying stroke risk factors and beginning stroke prevention therapies is an important aspect of the recovery plan. 

The outlook of cerebellar involvement of MS depends on the overall disease course, and symptoms may improve or can persist for the long term. 

Neurodegenerative diseases, including MSA and CJD, are expected to decline, and can eventually be fatal. 

Summary 

Cerebellar degeneration is a rare neurological effect of a diverse array of serious neurological diseases. It causes ataxia, coordination problems, and nystagmus.

Most of the time, cerebellar symptoms are diagnosed based on a physical examination. Imaging tests and blood tests can help identify the cause. Treatment often doesn’t reverse cerebellar degeneration, but it may slow progression and help control symptoms. 

A Word From Verywell 

Cerebellar degeneration is not common, and the causes are serious, so a diagnosis can be alarming and isolating.

Make sure you talk to your medical team about getting support such as physical therapy, occupational therapy, and additional caregiver support as needed. This can help you with your health needs and to figure out how to get around your home and other places safely.

It may also help to reach out to a support group so you can connect with others who are having the same experiences and challenges as you.